Autoimmune hepatitis and chronic biliary tract disease in childhood

Autoimmune hepatitis is a rare but severe childhood disease, the presentation can be acute, even fulminant. This propensity for an acute presentation is one of the distinguishing features of childhood autoimmune hepatitis along with an increased incidence of type 2 disease. The diagnosis of primary sclerosing cholangitis should be one of exclusion in childhood, accounting for secondary causes and mimics the pathologist should be aware of. In childhood, sclerosing cholangitis can arise in a setting mimicking autoimmune hepatitis both biochemically, serologically and histologically – ‘autoimmune sclerosing cholangitis’. When the pathologist is confronted with unexplained biliary features in a childhood biopsy the spectrum of differential diagnoses differs from the adult setting.